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《天津医科大学学报》[ISSN:1006-8147/CN:12-1259/R]

卷:

26卷

期数:

2020年01期

页码:

32-34

栏目:

临床医学

出版日期:

2020-04-06

文章信息/Info

Title:

Clinical features of connective tissue disease associated Pulmonary artery hypertension

文章编号:

1006-8147(2020)01-0032-03

作者:

王 慧¹; 张 娜¹; 赵 音¹; 杨振文²; 曹 洁³; 董丽霞³; 魏 蔚¹

（天津医科大学总医院1.风湿免疫科；2.心脏科；3.呼吸科，天津300052）

Author(s):

WANG Hui¹;  ZHANG Na¹;  ZHAO Yin¹;  YANG Zhen-wen²;  CAO Jie³;  DONG Li-xia³;  WEI Wei¹

（1.Department of Rheumatology and Immunology, 2. Department of Cardiology, 3.Department of Respiration, General Hospital, Tianjin Medical University, Tianjin 300052, China）

关键词:

结缔组织病; 动脉性肺动脉高压; 右心导管; 超声心动图

Keywords:

connective tissue disease; pulmonary artery hypertension; right heart catheterization; echocardiography

分类号:

R593.2

DOI:

-

文献标志码:

A

摘要:

目的：分析结缔组织病相关动脉性肺动脉高压（CTD-PAH）患者的临床特点，提高对疾病的认识。方法：回顾性总结经右心导管（RHC）确诊的70例CTD-PAH患者的临床资料,分析其特征。结果：患者均为女性，平均年龄（43.8±13.9）岁，最常见的PAH症状为活动后气短、乏力。33例（47.1%）有雷诺现象。常见的基础疾病为系统性红斑狼疮（SLE，48.6%）及原发性干燥综合征（pSS，20%）。抗SSA抗体阳性率（54.9%）较高。69例（98.6%）超声心动图结果提示可能存在肺动脉高压（PH），38例（54.9%）有心包积液。患者1年及3年的生存率为96%和93%。结论：多种CTD可继发PAH,以SLE及pSS多见。雷诺现象、抗SSA抗体及心包积液可能是CTD-PAH发病的危险因素。超声心动图是筛查CTD-PAH的有效检查。激素及免抑制剂治疗可能对于改善病情有积极作用。

Abstract:

Objective: To analyze the characteristics of patients with Connective tissue disease associated Pulmonary artery hypertension (CTD-PAH) patients for improving understanding of the disease. Methods: The 70 CTD-PAH patients diagnosed by right heart catheterization(RHC) were analyzed retrospectively. Results: All patients were female, age was (43.8±13.9)years.The common symptoms of PAHwere dyspneaand fatigue.The rate of Raynaud’s phenomenon was 47.1%. The main underlying disease were systemic lupus erythematosus(48.6%) and primary Sjogren’s syndrome(20%). The positive rate of anti-SSA antibody was 54.9%.The 98.6% of the echocardiographic resultssuggested there may bethe pulmonary hypertension.The 1-year and 3-year survival rates were 96% and 93%. Conclusion: Pulmonary artery hypertension can occur in many connective tissue diseases, SLE and pSS are common. Raynaud’s phenomenon, anti-SSA antibody and pericardial effusion may be risk factors of CTD-PAH.Echocardiography contributes to the screening of CTD-PAH. Glucocorticoid and immunosuppressive therapy may have a positive effect on improving the disease.

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相似文献/References:

[1]闫喆.主动循环呼吸技术对进展性纤维化性间质性肺病患者的治疗效果[J].天津医科大学学报,2022,28(04):414.
　YAN Zhe.Effect of active cycle of breathing technologies on patients with progressive fibrotic interstitial lung disease[J].Journal of Tianjin Medical University,2022,28(01):414.

备注/Memo

备注/Memo:

作者简介 王慧（1990-），女，住院医师，硕士，研究方向：风湿病；通信作者：魏蔚，E-mail:tjweiwei@163.com。

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更新日期/Last Update: 2020-04-16